Following up after GIFT of life
February 13, 2010
Diane Flacks
SPECIAL TO THE STAR
Dr. Paul Wales, 42, is a pioneer in duck boots, a scalpel-wielding maestro in lab coat and side part. The parent of a former patient simply refers to him as "a blessing."
In person, the general surgeon is preternaturally young, soft-spoken, his words interrupted by little breathy chuckles. The good humour is well-founded. He has made it his mission to try and save children who were once considered doomed.
The program Wales created in 2002 at Toronto's Hospital for Sick Children, GIFT (the Group for the Improvement of Intestinal Function and Treatment), helps those with minimal working bowel (short bowel syndrome or SBS). They were expected to die in early childhood, often of liver failure or complications from bowel transplants.
"One hundred years from now, people will look back and it will seem like we were putting leaches on people," Wales says of the tragic death rate. "We'll look so barbaric."
He credits his team's comprehensive and integrated response, new medications and new surgical procedures with cutting the mortality rate from liver failure from 22 per cent before GIFT was formed to 1.5 per cent in 2006-2007.
Stats like these should be cause for celebration, so why do they also keep Wales up nights?
Because, by law, when these kids turn 18, they have to leave Sick Kids and the GIFT program, Wales, and their medical "home." They must transfer to an adult medical system that didn't expect them to live. And they must negotiate it alone.
"It would be nice if there was an adult equivalent program that patients could step into," Wales says on the phone from his office at Sick Kids after a busy morning in the operating room. "We're a very hands-on team. Families get a lot of face time. It's emotional, because a lot of them (patients) started off as babies. You have to build that trust in adult medicine."
Parents have always been an integral part of care, Wales says, but once their child turns 18 they are cut out of decision-making.
"Because of privacy legislation and confidentiality, all of a sudden they're not even asked. The children have to make decisions on their own."
Not something that is easy to do the day you turn 18, never mind when you have complex medical conditions.
Wales' team is trying to do a better job of preparing SBS kids to make their own decisions, adapting a general hospital program called Good To Grow.
Because frequent hospitalizations combined with extra medical responsibilities prevent many Sick Kids' patients from hitting regular developmental markers, Good to Grow outlines milestones they should meet at various ages. Wales is adapting this system for SBS kids, while encouraging the adult system to be open to GIFT's information and innovation.
Wales is driven. Perhaps, he's also haunted. When he was a surgical fellow at the hospital, he was on call when a 28-week-old girl needed treatment for necrotizing enterocolitis, an acute infection that causes destruction of the bowel.
"We went to OR and opened the baby up. There were 20 centimetres of bowel remaining. I remember standing in the OR and asking, `How much gut is enough? When do we close and say there's nothing we can do and when do we go for it?' There were no answers."
There was no standard approach to patients like that baby, he explains. Some were sent to pediatrics, some to surgery. Some had transplants.
"They would all die of liver failure, a miserable death, just under a year, and would never be home. They would just die in the hospital."
That's what happened to the little girl in the OR. "I'll never forget that night," Wales says softly. "She just kept hemorrhaging and she just died. And you're totally helpless." At that moment, he decided, "we just have to do better than this."
Now, they are.
But the success has the unexpected consequence that patients who live into adulthood no longer have parental collaboration and support to help them maintain their health.
In 10 to 15 years, there will be a large cohort of these SBS patients "graduating" from Sick Kids.
With 10 years to prepare, will the adult and children's systems be functioning seamlessly as a team, ready to receive them?
In the future, he predicts, integrating the children's and adult medical system will seem just as much a no-brainer as the GIFT interventions seem now.
Dr. Erwin Oechslin, director of the Adult Congenital Heart Disease Clinic at the Peter Munk Cardiac Centre at the University Health Network, hopes that his department can serve as an example.
Oechslin explains over the phone that before the 1960s, many kids with congenital heart defects didn't survive. That changed thanks to medical advances, and a large group of youths entered the adult system in the '70s and '80s.
That's when cardiologists discovered that communication and close collaboration between pediatric and adult health care are crucial. Adult cardiologists now are trained in pediatric cardiology and the doctors from the Hospital for Sick Children go over to the University Health Network to form a team that consults and communicates during the transitional years.
"During childhood, the pediatric system invests a lot of money to help these kids survive," Oechslin says.
"Our mandate is: we must validate the investment that was made in these kids."
Toronto Star